ipv vs cpt for airway clearance during cf exacerbation

Conditions: Cystic Fibrosis; Airway Clearance Impairment

Summary: intrapulmonary percussive ventilation (ipv) will show greater improvements in forced expiatory volume in 1 second (fev1) shorter duration of stay, and greater time to next pulmonary exacerbation in pediatric cystic fibrosis patients admitted for pulmonary exacerbation. fev1 is a measurement of obstruction in the airway which is seen to be elevated in cf patients with worsening lung disease. fev1 is used as a primary endpoint in pulmonary trials because it is an acute indicator of worsening or improving lung function.

the study will be a prospective randomized clinical trial. all cystic fibrosis (cf) patients five years and above who are being admitted to university of florida (uf) shands children's hospital with the diagnosis of cf acute pulmonary exacerbations will be approached for study consent on day one of hospitalization. each patient will be randomized to receive either ipv (metaneb) or chest physiotherapy (cpt) vest (incourage) therapy. treatments will be administered by in-house respiratory therapists four times daily according to standard protocol, and the respiratory therapist will document each treatment in the patient's electronic medical record (epic). each patient will also receive mucolytic agents (dornase alpha, hypertonic saline) and antibiotic therapy as per routine inpatient protocol per the attending pulmonologist on service. if the patient's clinical status worsens during their admission the attending pulmonologist will be allowed to change the airway clearance regimen or antibiotic coverage at their discretion. if a patient is enrolled and has another admission for pulmonary exacerbation after their initial admission, we will attempt to randomize them to the other arm and utilize this data to see if there is a difference in outcome parameters for the same patient if they are randomized to both arms. the degree of improvement in fev1 defined as absolute difference between admission fev1 and discharge fev1 will be used as primary outcome measure to compare effectiveness between both modalities. this measure is commonly used in the cystic fibrosis community of clinicians and researchers as measure of airway obstruction and disease severity. we will perform spirometry at least twice, at admission and discharge, on these patients. the patient's spirometry from their office visit on the same day of admission or first spirometry during their admission will count as the initial spirometry. last spirometry obtained before discharge will be the discharge spirometry. additional spirometry may be performed during the patient's admission at the attending provider's clinical discretion. we will also use data from these additional spirometry measurements if they take place. there will be no additional cost incurred by using the ipv device as opposed to standard of care (cpt vest).there is also no increased effort performing ipv treatments from the respiratory therapist performing the treatment or from the patient. length of hospital stay in days will be used as a secondary outcome. another secondary outcome measure will be the length of time between discharge and subsequent admissions for cf pulmonary exacerbation. we will follow the subject for an additional 6 months after they are enrolled to ascertain the time to next admission and total number of admissions in that time. other information that will be accessed through epic records will be number of admissions for cf pulmonary exacerbations, airway clearance regimen, radiological data, laboratory results (respiratory cultures, respiratory viral panels, acid fast bacilli cultures, complete blood counts), vital signs, weight measurements, bronchoscopy reports, medications used (orkambi, kalydeco, antibiotics), and oxygen requirement. all of this information will be collected in the time period of 1 year before study and throughout study, we will also administer a 2 question questionnaire at the beginning and end of admission to the hospital in order to evaluate the patient/family opinion of the therapy they received during their participation in the study.

Eligibility criteria:
inclusion criteria: * cf patient admitted for pulmonary exacerbation exclusion criteria: * patients who are not able to perform spirometry, patients with neurological impairment (cerebral palsy) or severe behavioral problems that precludes consistent use of ipv, patients with pneumothorax or significant hemoptysis, patients who require supplemental oxygen or ventilatory support, or patients whose parents/guardians are not willing to consent will be excluded.